What is the long-term outlook for children with megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)?
Survival in MMIHS seems to have improved, thanks to more specialized care, innovations in parenteral nutrition, and introduction of multivisceral transplantation. Long-term survival usually requires total parenteral nutrition and urinary catheterization or diversion. Most long-term survivors have ileostomies. In families with an inherited MMIHS-causing mutation, some family members with a mutation have milder features, living into adolescence and early adulthood.
While there are reports of longer survival, the prognosis and life expectancy remains poor, and it is still fatal in many cases. The main causes of death include sepsis, malnutrition, or multiple organ failure.
Last updated: 8/10/2017
What is the age of the oldest reported person with megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)?
In a review of the literature published in 2011 including 227 cases of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), the oldest survivors were a 19 year old female and 24 year old male. However the 24 year old male was only diagnosed as an adult and appeared to have a milder case. The 19 year old female however was diagnosed shortly after birth. What seems special about this case is that she had been able to remain free of infections since the age of 4 or 5 years old. In the most recent case report in 2016 which reported on 4 people with MMIHS, one was a 20 year old female survivor as well.
Last updated: 10/31/2016
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Trebicka J, Biecker E, Gruenhage F, Stolte M, Meier-Ruge WA, Sauerbruch T, and Lammert F. Diagnosis of megacystis-microcolon intestinal hypoperistalsis syndrome with aplastic desmosis in adulthood: a case report. Eur J Gastroenterol Hepatol. April 2008; 20(4):353-355. https://www.ncbi.nlm.nih.gov/pubmed/18334881.
Talisetti A, Longacre T, Pai RK, and Kerner J. Diversion colitis in a 19-year-old female with megacystis-microcolon-intestinal hypoperistalsis syndrome. Dig Dis Sci. November 2009; 54(11):2338-40. https://www.ncbi.nlm.nih.gov/pubmed/19582576.
Wymer KM, Anderson BB, Wilkens AA, and Gundeti MS. Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management. J Pediatr Surg. September 2016; 51(9):1565-73. https://www.ncbi.nlm.nih.gov/pubmed/27421821.